Question 1

In addition to classic galactosemia, what are the other two variants of galactosemia?

Accepted Answer

A)  Duarte and Black 
B)  variant I and variant II 
C)  Edwards and Jackson 
D)  dairy and fruit 
E)  secondary and tertiary 
A)  Duarte and Black 
B)  variant I and variant II 
C)  Edwards and Jackson 
D)  dairy and fruit 
E)  secondary and tertiary A

Question 2

PD is an 11-month-old boy with phenylketonuria (PKU). His mother brought him to an outpatient nutrition center in the local pediatric hospital for evaluation.
Length: 28 inches Weight: 19 pounds (8.7 kg)
-Phe restriction should achieve optimal blood Phe levels of:

Accepted Answer

A)  20-30 μmol/L 
B)  65-105 μmol/L 
C)  120-360 μmol/L 
D)  400-600 μmol/L 
E)  650-820 μmol/L 
A)  20-30 μmol/L 
B)  65-105 μmol/L 
C)  120-360 μmol/L 
D)  400-600 μmol/L 
E)  650-820 μmol/L C

Question 3

Describe the acute nutritional interventions used for urea cycle disorders.

Accepted Answer

Initial treatment includes administration of intravenous fluids because many infants are dehydrated at presentation due to anorexia and poor oral intake. Overhydration should be avoided because most patients with urea cycle defects have some degree of cerebral swelling. Caloric supplementation should be maximized to reverse catabolism and nitrogen turnover. In addition to glucose, Intralipid administration can provide the much-needed kilocalories in a smaller volume. Complete protein restriction is recommended only for a 24- to 48-hour period in order to avoid depletion of essential amino acids, which would result in further protein catabolism and nitrogen release. If enteral feedings cannot be tolerated, parenteral nutrition (PN) should be started. When protein is reintroduced into the diet, a standard PN solution with a low percentage of amino acids may be used if PN is only needed for a short duration. If enteral feedings are not resumed quickly, then the use of a special PN solution rich in the essential branched-chain amino acids should be considered.

Question 4

How are most inborn errors of metabolism acquired?

Accepted Answer

A)  autosomal dominant inheritance 
B)  autosomal recessive inheritance 
C)  infection 
D)  dietary deficiency 
E)  blood toxicity 
A)  autosomal dominant inheritance 
B)  autosomal recessive inheritance 
C)  infection 
D)  dietary deficiency 
E)  blood toxicity

Question 5

What is the most predominant type of mitochondrial disorder?

Accepted Answer

A)  fatty acid transport disorders 
B)  fatty acid oxidation defects 
C)  glycogen complex disorders 
D)  respiratory chain disorders 
E)  pyruvate complex disorders 
A)  fatty acid transport disorders 
B)  fatty acid oxidation defects 
C)  glycogen complex disorders 
D)  respiratory chain disorders 
E)  pyruvate complex disorders

Question 6

Describe how clinicians screen for multiple metabolic disorders in infants.

Accepted Answer

Tandem mass spectroscopy allow

Question 7

Which amino acid is affected in the metabolic condition phenylketonuria (PKU)?

Accepted Answer

A)  methionine 
B)  leucine 
C)  tryptophan 
D)  phenylalanine 
E)  cysteine 
A)  methionine 
B)  leucine 
C)  tryptophan 
D)  phenylalanine 
E)  cysteine

Question 8

Isovaleric acidemia (IVA) is an amino acid disorder affecting the metabolism of multiple amino acids.

Accepted Answer

A) True 
 B)False

Question 9

Which of the following drugs binds with homocysteine to form the amino acid methionine?

Accepted Answer

A)  metronidazole 
B)  cystadane 
C)  amoxicillin 
D)  cyclosporine 
E)  tacrolimus 
A)  metronidazole 
B)  cystadane 
C)  amoxicillin 
D)  cyclosporine 
E)  tacrolimus

Question 10

Selective neonatal screening is the testing of an individual known to be at increased risk for a _______________ disorder.

Accepted Answer

The answer of Selective neonatal screening is the testing of...

Question 11

Some of the galactose in fruits and vegetables may be found as the galactose _______________, raffinose and stachyose, and hence is most likely unavailable for absorption.

Accepted Answer

The answer of Some of the galactose in fruits and...

Question 12

PD is an 11-month-old boy with phenylketonuria (PKU). His mother brought him to an outpatient nutrition center in the local pediatric hospital for evaluation.
Length: 28 inches Weight: 19 pounds (8.7 kg)
-What two common PKU characteristics did PD's mom likely notice in her son?

Accepted Answer

A)  fair complexion and musty-smelling urine 
B)  dark skin pigmentation and foul breath 
C)  bowed legs and high-pitched screeching 
D)  bloody diarrhea and muscle paralysis 
E)  severe insomnia and fatty stool 
A)  fair complexion and musty-smelling urine 
B)  dark skin pigmentation and foul breath 
C)  bowed legs and high-pitched screeching 
D)  bloody diarrhea and muscle paralysis 
E)  severe insomnia and fatty stool

Question 13

Which vitamin supplement is used as a treatment for the holocarboxylase synthetase deficiency disorder?

Accepted Answer

A)  thiamin 
B)  riboflavin 
C)  biotin 
D)  niacin 
E)  folic acid 
A)  thiamin 
B)  riboflavin 
C)  biotin 
D)  niacin 
E)  folic acid

Question 14

What is the term used for a substrate needed for the normal metabolism of fat for energy?

Accepted Answer

A)  carnitine 
B)  glucagon 
C)  raffinose 
D)  ribose 
E)  amylopectin 
A)  carnitine 
B)  glucagon 
C)  raffinose 
D)  ribose 
E)  amylopectin

Question 15

Inhibition of both _______________ and glycogenolysis results in fructose-induced hypoglycemia.

Accepted Answer

The answer of Inhibition of both _______________ and glycogenolysis results...

Question 16

In glycogen storage disease type III, protein is used as an alternative source of glucose production, via the _______________ shunt.

Accepted Answer

The answer of In glycogen storage disease type III, protein...

Question 17

Which enzyme deficiency results in the accumulation of fructose-1-phosphate?

Accepted Answer

A)  glucokinase 
B)  arginase 
C)  phenylalanine hydroxylase 
D)  lipase 
E)  aldolase B 
A)  glucokinase 
B)  arginase 
C)  phenylalanine hydroxylase 
D)  lipase 
E)  aldolase B

Question 18

What is the function of riboflavin and thiamin in the respiratory chain?

Accepted Answer

A)  antioxidants 
B)  cofactors 
C)  artificial electron receptors 
D)  artificial electron transporters 
E)  antivirals 
A)  antioxidants 
B)  cofactors 
C)  artificial electron receptors 
D)  artificial electron transporters 
E)  antivirals

Question 19

Pharmaceutical products such as sodium ________________ and sodium phenylbutyrate are needed to remove the excess nitrogen that is produced as a result of a block in the urea cycle.

Accepted Answer

The answer of Pharmaceutical products such as sodium ________________ and...

Question 20

What is a common secondary consequence of metabolic defects?

Accepted Answer

A)  bacterial infections 
B)  nutritional deficiencies 
C)  arthritis 
D)  cancer 
E)  autoimmune disorders 
A)  bacterial infections 
B)  nutritional deficiencies 
C)  arthritis 
D)  cancer 
E)  autoimmune disorders

Exam 25: Metabolic Disorders

In addition to classic galactosemia, what are the other two variants of galactosemia?

PD is an 11-month-old boy with phenylketonuria (PKU). His mother brought him to an outpatient nutrition center in the local pediatric hospital for evaluation. Length: 28 inches Weight: 19 pounds (8.7 kg) -Phe restriction should achieve optimal blood Phe levels of:

Describe the acute nutritional interventions used for urea cycle disorders.

How are most inborn errors of metabolism acquired?

What is the most predominant type of mitochondrial disorder?

Describe how clinicians screen for multiple metabolic disorders in infants.

Which amino acid is affected in the metabolic condition phenylketonuria (PKU)?

Isovaleric acidemia (IVA) is an amino acid disorder affecting the metabolism of multiple amino acids.

Which of the following drugs binds with homocysteine to form the amino acid methionine?

Selective neonatal screening is the testing of an individual known to be at increased risk for a _______________ disorder.

Some of the galactose in fruits and vegetables may be found as the galactose _______________, raffinose and stachyose, and hence is most likely unavailable for absorption.

PD is an 11-month-old boy with phenylketonuria (PKU). His mother brought him to an outpatient nutrition center in the local pediatric hospital for evaluation. Length: 28 inches Weight: 19 pounds (8.7 kg) -What two common PKU characteristics did PD's mom likely notice in her son?

Which vitamin supplement is used as a treatment for the holocarboxylase synthetase deficiency disorder?

What is the term used for a substrate needed for the normal metabolism of fat for energy?

Inhibition of both _______________ and glycogenolysis results in fructose-induced hypoglycemia.

In glycogen storage disease type III, protein is used as an alternative source of glucose production, via the _______________ shunt.

Which enzyme deficiency results in the accumulation of fructose-1-phosphate?

What is the function of riboflavin and thiamin in the respiratory chain?

Pharmaceutical products such as sodium ________________ and sodium phenylbutyrate are needed to remove the excess nitrogen that is produced as a result of a block in the urea cycle.

What is a common secondary consequence of metabolic defects?

Role of the Registered Dietitian Nutritionist in the Health Care System

Overview the Nutrition Care Process

Nutrition Assessment Foundation of the Nutrition Care Process

Nutrition Intervention Nutrition Monitoring and Evaluation

Enteral and Parenteral Nutrition Support

Nutrition Informatics and Documentation of the Nutrition Care Process

Fluid and Electrolyte Balance

Acid Base Balance

Cellular and Physiological Response to Injury the Role of the Immune System

Nutritional Genomics

Pharmacology

Diseases and Disorders of Energy Imbalance

Diseases of the Cardiovascular System

Diseases of the Upper Gastrointestinal Tract

Diseases of the Lower Gastrointestinal Tract

Diseases of the Liver Gallbladder and Exocrine Pancreas

Diseases of the Endocrine System

Diseases of the Renal System

Diseases of the Hematological System

Diseases and Disorders of the Neurological System

Diseases of the Respiratory System

Metabolic Stress and the Critically Ill

Neoplastic Disease

Diseases of the Musculoskeletal System

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